Clone Name: AT1E11
Light Chain: Kappa
Calculated MW: 54
Form: Liquid
Buffer (with preservative): PBS, 10% Glycerol, 0.02% Sodium azide.
Concentration: 1 mg/ml (Please refer to the vial label for the specific concentration.)
Background: The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. The PDH complex thereby links glycolysis to Krebs cycle. The PDH complex contains three catalytic subunits, E1, E2, and E3, two regulatory subunits, E1 kinase and E1 phosphatase, and a non-catalytic subunit, E3 binding protein (E3BP). This gene encodes the E3 binding protein subunit; also known as component X of the pyruvate dehydrogenase complex. This protein tethers E3 dimers to the E2 core of the PDH complex. Defects in this gene are a cause of pyruvate dehydrogenase deficiency which results in neurological dysfunction and lactic acidosis in infancy and early childhood. This protein is also a minor antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC eventually leads to cirrhosis and liver failure. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Oct 2009]
Uniprot ID: O00330
Antigen Species: Human
Immunogen: The clone AT1E11 is derived from hybridization of mouse F2 myeloma cells with spleen cells from BALB/c mice immunized with a recombinant human PDHX protein.
Purification: Protein A Purified
Conjugation: Unconjugated
Full Name: pyruvate dehydrogenase complex component X
English
Deutsch
